Abstract
Congenital transmesenteric internal hernia is a rare condition presenting as recurrent pain abdomen or acute intestinal obstruction. Preoperative diagnosis of internal hernia is very rare. Early intervention and surgical correction goes a long way in preventing high morbidity and mortality associated with cases of internal hernia. We report a case of congenital transmesenteric intra-abdominal hernia presented as acute abdomen with timely surgical intervention in the form of emergency laparotomy with untwisting and reduction of small bowel herniated through large congenital terminal ileal mesenteric defect and repair of mesenteric defect.